Craig, my husband of 13 years, has a particularly aggressive case of VHL. He has had ten cerebellar hemangioblastomas. All of his tumors progressed rapidly once diagnosed. We've been sweethearts since high school. I became a neurological nurse in part because of Craig -- to learn to be a good medical advocate for him, and to help find the best possible treatments.

The "routine" MRI was exactly like all of the others we had experienced over the years. There is the scheduling, the waiting for the appointment, the hour long scan, and then every VHL patient's favorite pastime -- awaiting the dreaded results.

As we played out all of the possible scenarios, we never imagined the words we would hear: "Craig has four new brain tumors." We had tried to prepare for one. We have become realistic about VHL over the years, but we always tried to remain cautiously optimistic. We had told ourselves we could once again deal with a tumor that could easily be surgically removed. Four, however, was not in our game plan.

Then the news went from bad to worse. The neurosurgeon explained that my husband's tumors were all located in inoperable areas of the brain, areas where it is extremely uncommon to develop a hemangioblastoma, even for patients with VHL. Three of the four tumors were supratentorial [in the cerebrum. VHL tumors are generally in the cerebellum or the brain stem.] His prognosis was slow neurologic decline and eventually he would die from the tumors. The doctor said that he could attempt to remove the tumors by surgical resection, but the surgery would definitely leave Craig with serious neurological damage. The doctor also informed us that stereotactic radiosurgery might be an option.

This news was not encouraging. Stereotactic radiosurgery had been recommended two years earlier in 1988. At that time I had spoken with several physicians across the country and found that stereotactic radiosurgery had been used to treat very few hemangioblastomas nationwide. What I learned in 1988 was that the success on hemangioblastoma was questionable, and in some cases the treatment might not have benefited the patients at all, but caused additional problems.

However, with an open mind and no other options, I launched again into massive research. Perhaps things had changed in two years. Thankfully, it seemed they had! I now learned there are a few major medical institutions that have used both linear accelerators and gamma knife stereotactic radiosurgery for treatment of hemangioblastomas.

Their greatest success with stereotactic radiosurgery had been treatment of AVMs [vascular lesions]. Since hemangioblastomas are vascular tumors, I reasoned we had a good chance of success. Although the number of treated hemangioblastomas in 1990 were few, the outcomes reported to me were more encouraging. In some cases the tumors had actually decreased in size. In other cases they remained stable (with no increase in size). Most important, though, in most cases the symptoms produced by the tumors subsided.

Stereotactic radiosurgery is a little bit like laser surgery, done with radiation. First the doctors put a halo frame or helmet on the patient and fix it to the head. After this is complete, the patient has a CT, MRI, or angiogram with the helmet in place. Once the exact size and location of the tumor is determined, a computer is used by a team of neurosurgeons, radiation oncologists, and radiation physicists to determine the exact dose of radiation needed to treat the lesion.

Then the patient is taken to the linear accelerator or gamma knife unit. The helmet or halo is secured to the machine to keep the patient's head motionless. As many as 201 individual beams of radiation are aimed at the tumor site from different angles. The "surgery" occurs where the beams meet, directly at the target site. While the tissue each beam passes through receives only the one-beam dose, the tumor receives the total dosage from all directions.

The procedure may last from 10 minutes to one hour depending on the dose and location, or if more than one area is being treated in the same session. In Craig's case two tumors were treated in two separate sessions.

The patient usually spends the night in the hospital and is discharged the next day. This certainly was a welcome change to the very long hospital stays Craig had experienced on previous occasions!

There are still no published articles on stereotactic radiosurgery for treatment of hemangioblastoma, though I understand that there should be one or two articles appearing soon. I spoke with many experts nationwide concerning stereotactic radiosurgery for treatment of hemangioblastoma.

Physicians with expertise in VHL still believe that, where possible, microsurgery to remove the tumor remains the best treatment option for hemangioblastoma.

It is important to consider the potential side effects of stereotactic radiosurgery. Patients may experience nausea or headaches the day after treatment. However these discomforts usually subside quickly. There is the possibility of brain edema [swelling] around the tumor site, even months after treatment. This can be treated with steroids.

Most seriously, there is the possibility of radiation damage to surrounding brain tissue. Every precaution is taken to avoid this, but the possibility does exist and is important to consider when making a decision. This complication did occur in Craig's case. One of his tumors was close to the optic tract, and he developed a loss of peripheral vision two years after treatment. Each time there is additional treatment in a given area, there is increased risk of damage.

Usually best results of stereotactic radiosurgery are with solid tumors 2.5 cm or smaller. Doctors commonly follow small, asymptomatic hemangioblastomas on MRI scans. Many patients have small tumors which do not change for years. As long as they remain small and do not cause problems, there may be no need for action.

It is important to remember that every case is different. You need to discuss your own treatment options, and the best treatment for your particular case, with physicians you trust.

Cysts are frequently associated with hemangioblastomas. The physicians I spoke with believe stereotactic radiosurgery is best suited for the solid tumors, and that the patient would be best served to treat the cyst prior to radiosurgery. In Craig's case, this was accomplished by aspirating fluid and injecting P32.

P32 was described to me by neurosurgeon Dr. Haring J. W. Nauta as "a radioactive material which decays quickly and penetrates tissue a very short distance. It acts on the cells in the lining of the cyst and doesn't get into the body."

This procedure worked well for Craig's particular kind of cyst. There are other ways of treating cysts -- again, talk with your physician about the best treatment plan for you.

Even with all of these considerations, the physicians I spoke with believe stereotactic radiosurgery has a place in the treatment of VHL hemangioblastomas, especially for those otherwise considered inoperable. They also agree that more experience is needed with hemangioblastomas before we will know all of the benefits. Dr. Tomasz Helenowski of the Chicago Neurosurgical Center has had very promising early results. Dr. L. Dade Lunsford and Dr. George Alvaro at Presbyterian University Hospital in Pittsburgh have had similar good results in four of their eight patients, but four others have had recurrence of the lesions. All of the treatment centers I contacted reported similar results. It also appears that once the hemangioblastoma is significantly impaired, the associated cyst is also controlled.

The total number of hemangioblastomas treated with stereotactic radiosurgery remains statistically small. The physicians agree that more data needs to be collected before this treatment can be considered a cure for hemangioblastomas.

I can only say that in our own case, stereotactic radiosurgery was the best option, even with the complications. Craig's vision seems to be improving and may get back to where it was before the treatment. We have experienced VHL at its most cruel, and welcomed stereotactic radiosurgery as a treatment option. It provided us with hope when no hope was to be found.

I am happy to report that Craig's most recent scan (June 1993) is stable, which is very good news, and we are very grateful. He walks with only modest balance problems from prior episodes, speaks well, and drives. He will continue to pursue his Master's degree in pastoral counseling in the fall.

We would like to thank the entire medical team at Johns Hopkins Hospital for their compassion and concern during that difficult time. Special thanks go to Ben Carson, M.D.; Haring J.W. Nauta, M.D.; Moody D. Wharam, M.D.; Carol James, P.A., and Jennifer Bucholtz, R.N.

Many thanks to the physicians who have generously given me their time and helped immensely with the writing of this article, especially Dr. Tomasz Helenowski, Dr. George Alvaro, Dr. L. Dade Lunsford, Dr. Haring Nauta, Dr. Jay Loeffler and Dr. Moody Wharam. Special thanks to Dr. Lars Leksell who pioneered stereotactic radiosurgery at the Carolinska Institute in Stockholm, Sweden.

[Note 12/97: Please be sure to review with your medical team our Handbook and our list of Questions to Ask before proceeding with treatment. If your team does not have extensive experience in treating hemangioblastoma, we recommend that you have a more experienced center review the treatment plan before proceeding. Stereotactic radiosurgery is still surgery, and serious complications can ensue if the treatment plan is not optimal.]

Please let us know if you have had this treatment and would be willing to share your experience with researchers trying to determine the effectiveness of stereotactic radiosurgery in treating VHL. [See also the Report of the Focus Conference on Stereotactic Radiosurgery and VHL, May 1997.]

A List of Centers in the U.S. which perform stereotactic radiosurgery with gamma knife machines can be obtained from the American Brain Tumor Association, 2720 River Road, Des Plaines, IL 60018. Tel: 800-886-2282, Fax: 708-827-9918. The Alliance will helpt you locate a center near you which performs stereotactic radiosurgery with linear accelerator or gamma knife machines.

References: American Brain Tumor Association newsletter, The Message Line, Spring 1989, "Research Update: Stereotactic Gamma Knife Radiosurgery". Jennifer Bucholtz, RN, "Stereotactic Radiosurgery, Standard Teaching Plan," Johns Hopkins Hospital, 1992. Griff Harsh, MD, and David A. Larson, MD, "Gamma Knife Treats Brain Lesions," University of California San Francisco Clinical Profile 4:1 (1992). Jay S. Loeffler, M.D., Eben Alexander II, MD, "The Role of Stereotactic Radiosurgery in the management of Intercranial Tumors," Oncology 4:3 (March 1990), 21-38. L. Dade Lunsford, "Stereotactic Radiosurgery: At the Threshhold or at the Crossroads," Neurosurgery, 32:5 (May 1993) 799-804. Haring J. W. Nauta, MD, Ph.D., M. Waseem Farra, MD, and Moody D. Wharam, MD, article in preparation for publication, "The Role of Stereotactic Radiosurgery in the Treatment of CNS Hemangioblastomas Associated with von Hippel-Lindau Syndrome."

As published in the VHL Family Forum 1:3, September 1993. For permission to reprint, please contact the VHL Family Alliance at editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.